RESUMO
Herein, we present a rare case of a nine-month-old boy diagnosed with infant-type hemispheric glioma (gliosarcoma subtype) at the left frontal lobe. Following subtotal resection, the patient started chemotherapy with the BABY POG protocol. We describe the clinical diagnosis, histological characteristics, radiological features, molecular aspects, and management of this tumor. A comprehensive molecular analysis on the tumor tissue showed a TPR-NTRK1 gene fusion. The patient was treated with a TRK inhibitor, larotrectinib, and exhibited a stable disease with residual lesion following 8 months of target therapy. The present study is the first report of an infantile gliosarcoma harboring NTRK1 rearrangement treated with larotrectinib.
Assuntos
Astrocitoma , Glioma , Gliossarcoma , Glioma/tratamento farmacológico , Glioma/genética , Gliossarcoma/tratamento farmacológico , Humanos , Lactente , Masculino , Pirazóis/uso terapêutico , Pirimidinas , Receptor trkA/genéticaRESUMO
The relative frequency of pediatric gliosarcoma (GSM) is 1.9% among glioblastomas and 0.5% among pediatric central nervous system tumors. A 5-year-old female child came to us with history of fever and loss of appetite since 2 weeks and right sided weakness since 4 days. Magnetic resonance imaging showed a large heterogeneously enhancing space occupying lesion in the left parieto-occipital region. A parieto-occipital craniotomy with radical excision of tumor was performed. The patient was given adjuvant therapy following surgery and survived until 9 months following surgery. The etiopathogenesis, treatment modalities and prognosis of GSM is discussed.